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Annular Pancreas Case Conference SUNY Downstate May 19, 2011
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Case • HPI: 10 month old female with a 3 week history of intermittent non-bilious projectile vomiting. No F/C, diarrhea, sick contacts. • PMH/PSH: None. • FH: Mother with SC trait. • SH: Only child to healthy parents. • ROS: FTVD, immunizations UTD
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Case • PE – VS: 98.5, 134, 91/44, 26 – WDWN, NAD – Ht: 30 in (90 %ile); Wt 20 lbs (50 %ile) – NCAT – RRR, no MGR – CTAB – SNTND, no masses – Bilious vomiting
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Case • Labs – CBC: 13 / 12.1 / 35.6 / 471 – BMP: 129 / 3.0 / 88 / 18.4 / 26 / 0.7
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Case
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Case
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Case
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Case • • • •
Resuscitated. Electrolyte abnormalities corrected. Underwent duodenojejunostomy. PEG and feeding jejunostomy tube placed.
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Case
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Case
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Case • • • • •
POD 2 – TPN started POD 5 – Feed via J-tube POD 9 – Oral diet POD 12 – PEG clamped POD 14 – discharged to home
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ANNULAR PANCREAS
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Definition • Ring of pancreatic tissue surrounding the descending portion of the duodenum. • May be complete or partial.
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Embryology • Adherence of the ventral bud to the duodenal wall prior to rotation resulting in its persistence and encirclement of the duodenum (Lecco's theory). • Persistence and enlargement of the left ventral bud (Baldwin's theory). • Hypertrophy and fusion of the ventral and dorsal buds before rotation of the gut resulting in complete encirclement of the duodenum
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Embryology
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Epidemiology • Estimated incidence – 3 in 20,000 to 1 in 1,000
• 33% asymptomatic • 50% pediatric presentation – 86% neonatal period
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Presentation • Prenatal – Maternal polyhydroamnios
• Children – bilious vomiting – feeding difficulties – upper abdominal distention
• Adults – abdominal pain – gastric outlet obstruction – pancreatitis, PUD, obstructive jaundice
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Diagnosis • • • • • • •
Abdominal XR or U/S – “Double bubble” UGI series CT scan ERCP EUS MRCP Surgery
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AXR
Traubici, et al. The Double Bubble Sign. Radiology. 2001. 220: 463-464.
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U/S
Traubici, et al. The Double Bubble Sign. Radiology. 2001. 220: 463-464.
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Double Bubble Intrinsic • Duodenal atresia • Duodenal stenosis • Duodenal webs
Extrinsic • Annular pancreas • Malrotation • Ladd Bands • Preduodenal position of portal vein
Traubici, et al. The Double Bubble Sign. Radiology. 2001. 220: 463-464.
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ERCP
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MRCP
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CT Scan
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CT / MRI Findings
Sandrasegaran, et al. Annular Pancreas in Adults. Am J Roent. 2009 193: 455-460.
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Treatment • Bypass operation – Duodenoduodenostomy – Duodenojejunostomy – Gastrojejunostomy
• No resection of annulus • Resection associated with: – Pancreatitis – Pancreatitc fistula formation – Incomplete relief of symptoms
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Operative Technique
Lukish, et al. Operative Techniques in General Surgery. 2002. 4,2: 135-140.
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Operative Technique
Lukish, et al. Operative Techniques in General Surgery. 2002. 4,2: 135-140.
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Operative Technique
Lukish, et al. Operative Techniques in General Surgery. 2002. 4,2: 135-140.
www.downstatesurgery.org
Operative Technique
Lukish, et al. Operative Techniques in General Surgery. 2002. 4,2: 135-140.
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Operative Technique
Lukish, et al. Operative Techniques in General Surgery. 2002. 4,2: 135-140.
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Comparison of Annular Pancreas in Children and Adults 60 50
Number
40 30 20 10 0 0-10
11-20
21-30
31-40
41-50
51-60
61-70
71-80
Age [years] Zyromski, et al. Annular Pancreas: Dramatic Differences Between Children and Adults. J Am Col Surgeons. 2008. 206:5. 1019-1027.
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Presenting Symptoms Children
Adults n
%
n
%
Prenatal
47
56
Pain
41
75
GI
18
38
GI
13
24
Unknown
3
6
Pancreatitis
12
22
Jaundice
6
11
Unknown
3
6
Zyromski, et al. Annular Pancreas: Dramatic Differences Between Children and Adults. J Am Col Surgeons. 2008. 206:5. 1019-1027.
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Diagnosis Children
Adults n
%
n
%
AXR
30
63
ERCP
26
47
UGIS
9
19
CT
10
18
Ultrasound
3
6
MRCP
9
16
Unknown
6
12
Operative
7
13
Unknown
3
6
Zyromski, et al. Annular Pancreas: Dramatic Differences Between Children and Adults. J Am Col Surgeons. 2008. 206:5. 1019-1027.
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Congenital Anomalies Children
Adults n
%
n
%
Trisomy 21
10
21
Malrotation
3
5
Cardiac
10
21 21
Duodenal Web
2
4
Malrotation
10
Other GI
5
10
Shatzki ring
2
4
GU
5
10
1
2
TE fistula
4
8
Duodenal atresia
Other
5
10
TE fistula
1
2
Total
34
71
GU
1
2
Total
9
16
Zyromski, et al. Annular Pancreas: Dramatic Differences Between Children and Adults. J Am Col Surgeons. 2008. 206:5. 1019-1027.
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Neoplasia n
%
Pancreatic cancer
2
4
Ampullary adenoma
2
4
Gallbladder cancer
1
2
IPMN
1
2
Breast
3
5
Prostate
3
5
Leukemia/lymphoma
2
4
Lung
1
2
Colon
1
2
Ovary
1
2
Melanoma
1
2
13
24
Total Zyromski, et al.
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Therapy Children
Adults n
%
Duodenal bypass
48
100
Other congenital anomalies
20
42
n
%
Endoscopic
37
67
Cholecystec tomy
31
56
Duodenal bypass
13
24
Pancreas
8
14
Other congenital anomalies
5
9
Biliary
4
7
Zyromski, et al. Annular Pancreas: Dramatic Differences Between Children and Adults. J Am Col Surgeons. 2008. 206:5. 1019-1027.
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Laparoscopic Approach Open n = 14
Laparoscopic n = 15
p
96.3 min
126.7 min
0.06
Postop hospital stay
20.1 d
12.9 d
0.01
Time to initial feeding
11.3 d
5.4 d
0.002
Time to full oral intake
16.9 d
9.0 d
0.007
Operative Time
Spilde, et al. Open vs laparoscopic repair of congenital duodenal obstructions: a concurrent series. J Ped Surg. 2008. 43:6. 1002-1005.
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Take-aways • Clinical manifestations of annular pancreas can develop at any age. • In both children and adults with symptomatic annular pancreas, surgical bypass is indicated. • Patients with annular pancreas presenting obstructive jaundice require workup for periampullary malignancy.
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References • • • • • • • • •
Spilde, et al. Open vs laparoscopic repair of congenital duodenal obstructions: a concurrent series. J Ped Surg. 2008. 43:6. 1002-1005. Zyromski, et al. Annular Pancreas: Dramatic Differences Between Children and Adults. J Am Col Surgeons. 2008. 206:5. 1019-1027. Lukish, et al. Operative Techniques in General Surgery. 2002. 4,2: 135-140. Sandrasegaran, et al. Annular Pancreas in Adults. Am J Roent. 2009 193: 455-460. Rothenberg, et al. Laparoscopic Duodenoduodenostomy for Duodenal Obstruction in Infants and Children. J Ped Surg. 2002. 37:7. 1088-1089. Kay, et al. Laparoscopic duodenoduodenostomy in the neonate. J Ped Surg. 2009. 44. 906-908. Traubici, et al. The Double Bubble Sign. Radiology. 2001. 220: 463-464. Skandalakis, et al. "Chapter 21. Pancreas" (Chapter). Skandalakis' Surgical Anatomy: http://www.accesssurgery.com/content.aspx?aID=79125. Thukral, et al. Annular Pancreas. UpToDate: http://www.uptodate.com.newproxy.downstate.edu/contents/annularpancreas?source=search_result&selectedTitle=1~12.